Perfusion strategies in rare diseases: Antiphospholypid Syndrome.

Abstract

Introduction: The antiphospholipid syndrome is considered as a rare diseases, it is an autoimmune disease characterized by the formation of thrombus. Among the criteria for establishing the diagnosis are: venous and arterial thrombosis, recurrent pregnancy loss and the presence of antiphospholipid antibodies
Objectives: The treatment of patients with antiphospholipid syndrome in cardiac surgery with extracorporeal circulation represents a challenge in terms of anticoagulation strategy since this syndrome, although associated with thrombocytopenia and deficiencies of coagulation factors, is characterized by the formation of thrombus triggered by situations such as the extracorporeal circuit among others, anticoagulation and monitoring becomes complex since the coagulation cascade is altered at multiple points.
Methods: We present a case of a 30-year-old patient with antiphospholipid syndrome, endocarditis of the aortic valve, previous cerebrovascular accident. Plan: Aortic valve replacement. Heparinization was administered prior, postoperatively and in the extracorporeal circulation, constant monitoring of coagulation status was performed with activated clotting time and synergistically with thromboelastogram.
Results: A higher concentration of heparin than usual was necessary for extracorporeal circulation. Reversal of heparin was achieved with unusual minimum doses of protamine sulfate. Patient left the hospital at day 6 with no complications and no evidence of thrombus formation.
Conclusion: Preparation and consultation guided the strategies adopted in perfusion behavior to achieve optimal management of anticoagulation during cardiovascular trans-surgery in this challenging pathology in which the surgical team, especially the perfusionist, may be faced.